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AAP ZIKA ECHO (EXTENSION FOR COMMUNITY HEALTHCARE OUTCOMES)

AAP ZIKA ECHO Zika ECHO_Congenital... · V IRUS D ISEASE * (J ... – Microcephaly can occur as a result ... Galang RR, et al. Pregnancy Outcomes After Maternal Zika Virus Infection

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  • AAP ZIKA ECHO(EXTENSION FOR COMMUNITY

    HEALTHCARE OUTCOMES)

  • HOUSEKEEPING ITEMS For educational and quality improvement purposes, this ECHO session will be recorded Project ECHO collects participation data for each ECHO session. This data allows Project

    ECHO to measure, analyze, and report on the ECHO movements reach. Data is used in reports, on maps and visualizations, for research, for communications and surveys, for data quality assurance activities, and for decision-making related to new initiatives.

    To protect patient privacy, please do not provide any (PHI) protected health information. Please mute your microphone when not speaking. If you have video capability, please

    enable it. There is a chat function in Zoom that may be used to send messages to the group. For IT

    help, please chat to the AAP Admin and we will assist you.

  • TODAYS LECTURECongenital Abnormalities

    Steve Caddle, MD, MPH, FAAPAssistant Professor of Pediatrics at CUMCDivision of Child and Adolescent Health

    New York-Presbyterian Hospital/Columbia University Medical CenterWashington Heights Family Health Center

    Presented March 19, 2018

  • CASE DEFINITION CONGENITAL ZIKA VIRUS DISEASE* (JUNE 2016)

    Clinical Criteria

    Liveborn infant with congenital microcephaly, or intracranial calcifications, or structural brain or eye abnormalities, or other congenital central nervous system-related abnormalities not explained by another etiology.

    *Called Zika Virus Syndrome in some places and Zika Virus Disease others

    Source: https://wwwn.cdc.gov/nndss/conditions/zika/case-definition/2016/06/

    https://wwwn.cdc.gov/nndss/conditions/zika/case-definition/2016/06/

  • CASE CLASSIFICATION: PROBABLEA neonate meets clinical criteria for congenital disease; ANDThe neonates mother has an epidemiologic linkage or meets laboratory criteria for recent ZIKV or flavivirus infection; ANDThe neonate has laboratory evidence of ZIKV or flavivirus infection by:

    Positive ZIKV IgM antibody test of serum or CSF collected within 2 days of birth; AND positive neutralizing antibody titers against ZIKV and dengue or other

    flaviviruses endemic to the region where exposure occurred; OR negative dengue virus IgM antibody test and no neutralizing antibody

    testing performed.

    Source: https://wwwn.cdc.gov/nndss/conditions/zika/case-definition/2016/06/

    https://wwwn.cdc.gov/nndss/conditions/zika/case-definition/2016/06/

  • CASE CLASSIFICATION: CONFIRMEDA neonate meets the clinical criteria for congenital disease AND meets one of the following laboratory criteria:

    ZIKV detection by culture, viral antigen, or viral RNA in fetal tissue, umbilical cord blood, or amniotic fluid; or neonatal serum, CSF, or urine collected within 2 days of birth; OR

    Positive ZIKV IgM antibody test of umbilical cord blood, neonatal serum or CSF collected within 2 days of birth with positive ZIKV neutralizing antibody titers and negative neutralizing antibody titers against dengue or other flaviviruses endemic to the region where exposure occurred.

    Source: https://wwwn.cdc.gov/nndss/conditions/zika/case-definition/2016/06/

    https://wwwn.cdc.gov/nndss/conditions/zika/case-definition/2016/06/

  • Microcephaly is a very specific diagnosis, and typically unusual as an isolated finding: initially seen in newborns On ultrasound, typically defined as HC < 3rd %ile for GA

    Microcephaly became an early trigger to search for Zika association, but spectrum of disease became apparent Microcephaly can occur as a result of a fetal brain disruption sequence: this

    appears to be pathology of Zika infection

    MICROCEPHALY: THE MOST APPARENT MARKER?

  • SEVERE MICROCEPHALY WITH PARTIALLY COLLAPSED SKULL

    Source: http://www.bbc.com/news/world-latin-america-35584953

    http://www.bbc.com/news/world-latin-america-35584953

  • Zika virus intrauterine infection causes fetal brain abnormality and microcephaly: tip of the iceberg?

    Source: Ultrasound in Obstetrics & GynecologyVolume 47, Issue 1, pages 6-7, 5 JAN 2016 DOI: 10.1002/uog.15831http://onlinelibrary.wiley.com/doi/10.1002/uog.15831/full#uog15831-fig-0002

    http://onlinelibrary.wiley.com/doi/10.1002/uog.2016.47.issue-1/issuetochttp://onlinelibrary.wiley.com/doi/10.1002/uog.15831/full#uog15831-fig-0002

  • FETAL BRAIN ANOMALIES

    Microcephaly Hydrocephalus/hydranencephaly Absent structures: (CC, pons, cerebellar vermis) Neuronal migration disorders (lissencephaly) Fetal brain disruption sequence Cerebral calcifications Brain asymmetry

  • Shapiro-Mendoza CK, Rice ME, Galang RR, et al. Pregnancy Outcomes After Maternal Zika Virus Infection During Pregnancy U.S. Territories, January 1, 2016April 25, 2017. MMWR Morb Mortal Wkly Rep 2017;66:615-621. DOI: http://dx.doi.org/10.15585/mmwr.mm6623e1.

    http://dx.doi.org/10.15585/mmwr.mm6623e1

  • STUDY RESULTS SUMMARY Overall, about 5% of fetuses and infants born to women with lab evidence

    of Zika virus infection in the U.S. territories has possible Zika-associated birth defects Same as percentage reported in 50 U.S. states during 2016

    Possible Zika-associated birth defects including brain abnormalities and/or microcephaly were reported following Zika virus infection during every trimester of pregnancy

    Among completed pregnancies with positive nucleic acid tests confirming Zika virus infection identified in first, second and third trimesters, the percentage of fetuses or infants with possible Zika-associated birth defects was 8%, 5% and 4%, respectively

    Shapiro-Mendoza CK, Rice ME, Galang RR, et al. Pregnancy Outcomes After Maternal Zika Virus Infection During Pregnancy U.S. Territories, January 1, 2016April 25, 2017. MMWR Morb Mortal Wkly Rep 2017;66:615-621. DOI: http://dx.doi.org/10.15585/mmwr.mm6623e1.

    http://dx.doi.org/10.15585/mmwr.mm6623e1

  • KNOWN ABNORMALITIES UNIQUE TO ZIKA VIRUS SYNDROMECHARACTERIZING THE PATTERN OF ANOMALIES IN CONGENITAL ZIKA SYNDROME FOR PEDIATRIC CLINICIANS

    JAMA PEDIATRICS, OCTOBER 2016

    Severe microcephaly with partially collapsed skull Thin cerebral cortices with subcortical calcifications Macular scarring and focal pigmentary retinal mottling Congenital contractures Marked early hypertonia and symptoms of extrapyramidal involvement

    Source: http://jamanetwork.com/journals/jamapediatrics/fullarticle/2579543

    http://jamanetwork.com/journals/jamapediatrics/fullarticle/2579543

  • Fig 3 Severe microcephaly.

    Maria de Fatima Vasco Aragao et al. BMJ 2016;353:bmj.i1901

    2016 by British Medical Journal Publishing Group

  • VENTRICULAR CALCIFICATION OR OTHER CENTRAL NERVOUS SYSTEM LESIONS

  • CT Scans Reveal Extensive Abnormalities

    Source: Hazin et al, NEJM April 6, 2016

    Intracranial calcifications Global cortical hypogyration Ventriculomegaly Global cerebellar hypoplasia

    23 infants with microcephaly in Pernambuco, Brazil

  • OPHTHO CRITERIA FOR CZS

    First ophthalmological examination has to be performed within 30 days of birth.

    SIGNS: Focal pigment mottling of the retina and circular lesions of chorioretinal atrophy including the macula, along with optic nerve abnormalities Identified in 30% of newborns with microcephaly (Frietas et al, JAMA Ophtho 2/16)

  • MACULAR SCARRING AND FOCAL PIGMENTARY RETINAL MOTTLING

    Source: http://jamanetwork.com/journals/jamaophthalmology/fullarticle/2491896

    Fundus Photographs of a 2-Month-Old GirlThe right eye has granular, pigmentary mottling in the macula (A), and the left eye has a chorioretinal lobulated atrophic lesion and slight pigmentary mottling (B).

    http://jamanetwork.com/journals/jamaophthalmology/fullarticle/2491896

  • CONGENITAL CONTRACTURES (ARTHROGRYPOSIS)

    Arthrogryposis multiplex congenita (AMC), or simply arthrogryposis, describes congenital joint contractures in two or more areas of the body. It derives its name from Greek, literally meaning "curving of joints" (arthron, "joint"; grpsis, late Latin form of late Greek grpsis, "hooking").

    Source: https://en.wikipedia.org/wiki/Arthrogryposis

    https://en.wikipedia.org/wiki/Arthrogryposis

  • CONGENITAL CONTRACTURES (ARTHROGRYPOSIS)

    Source: http://www.bmj.com/content/354/bmj.i3899

    (A) Contracture in flexion of knee;

    (B) hyperextension of knee (knee dislocation);

    (C) clubfeet; (D) deformities in 2nd, 3rd,

    and 4th fingers; (E) joint contractures in legs

    and arms, without involvement of trunk

    http://www.bmj.com/content/354/bmj.i3899

  • MARKED EARLY HYPERTONIA AND SYMPTOMS OF EXTRAPYRAMIDALINVOLVEMENT

    Loss of upper motor and alpha motor neurons can result in hypertonia, or increase in muscle tone.

    Increased resistance is apparent when the arms and legs are extended.

    Hyperextension of the back and tightly clenched fists are often seen.

    Sources: http://neuroscience.uth.tmc.edu/s3/chapter06.html and http://www.slideshare.net/peso88888/neonatal-examination-45813957

    http://neuroscience.uth.tmc.edu/s3/chapter06.htmlhttp://www.slideshare.net/peso88888/neonatal-examination-45813957

  • OTHER KNOWN ABNORMALITIES ASSOCIATED WITH ZIKA VIRUSSYNDROME

    Strabismus Cutis Gyrata Hypotonia Hyperreflexia Glaucoma Irritability Seizures

  • OTHER OCULAR FINDINGS: STRABISMUS

    Increased occurrence of strabismus (crossed eyes) 14% in babies with congenital

    Zika virus syndrome in the first year of life, versus 3-5% in the general population.

    Source: Study by Marcia Tartarella, MD, PhD, et al, under review

  • CUTIS GYRATA

    Cutis verticis gyrata (CVG) is a descriptive term for a condition of the scalp manifesting as convoluted folds and furrows formed from thickened skin of the scalp resembling cerebriform pattern.

    Source: http://emedicine.medscape.com/article/1113735-overview and http://www.americorpshealth.biz/turner-syndrome/info-php.html

    http://emedicine.medscape.com/article/1113735-overviewhttp://www.americorpshealth.biz/turner-syndrome/info-php.html

  • HYPOTONIA

    Source: https://englishforphysio.wordpress.com/tag/spasticity/

    https://englishforphysio.wordpress.com/tag/spasticity/

  • HYPERREFLEXIA

    Hyperreflexia, or over-activity of the Autonomic Nervous System is defined as overactive or overresponsive reflexes. Examples of this can include twitching or spastic tendencies, which are indicative of upper motor neuron disease as well as the lessening or loss of control ordinarily exerted by higher brain centers of lower neural pathways (disinhibition).

    Source: http://calder.med.miami.edu/pointis/automatic.html

    http://calder.med.miami.edu/pointis/automatic.html

  • SEIZURESStudy in Brazil showed children with CZS presented a high incidence of epileptic seizures before the end of the first year of life. In a case series of Brazilian infants with CZS or +Zika-IgM:

    38% (n=40) had epileptic seizures 43.3% characterized as spasms 22.7% as generalized tonic-clonic seizures 20.5% as partial seizures 4.5% as other types of seizures

    Alves LV, Cruz DDCS, van der Linden A, et.al. (2016). Epileptic seizures in children with congenital Zika virus syndrome. Revista Brasileira de Sade Materno Infantil, 16(Suppl. 1), S27-S31. https://dx.doi.org/10.1590/1806-9304201600s100003

    https://na01.safelinks.protection.outlook.com/?url=https://dx.doi.org/10.1590/1806-9304201600s100003&data=02|01|[email protected]|2e08483b4c4d44f5bed808d58d30aff8|686a5effab4f4bad8f3a22a2632445b9|0|0|636570162612497896&sdata=TQp1TLR6NbQ3bFmfV2umQb3lWq4zy/3zlVJ/t/OBZKQ%3D&reserved=0

  • SUMMARY: ZIKA- ASSOCIATED ADVERSEPREGNANCY OUTCOMES

    Fetal loss/miscarriage, stillbirth Fetal brain anomalies

    Microcephaly Ventriculomegaly Intracranial calcifications

    Eye abnormalities Neurologic

    Hypertonia Arthrogryposis Seizures

  • QUESTIONS?

    AAP Zika ECHO (Extension for Community Healthcare Outcomes)Housekeeping itemsTodays LectureCase definition Congenital Zika Virus Disease* (June 2016)Case classification: ProbableCase classification: ConfirmedMicrocephaly: the most apparent marker?Severe microcephaly with partially collapsed skullZika virus intrauterine infection causes fetal brain abnormality and microcephaly: tip of the iceberg?Fetal Brain AnomaliesSlide Number 11Study Results SummaryKnown Abnormalities Unique to Zika Virus Syndrome Characterizing the Pattern of Anomalies in Congenital Zika Syndrome for Pediatric Clinicians JAMA Pediatrics, October 2016Slide Number 14ventricular calcification or other central nervous system lesionsCT Scans Reveal Extensive AbnormalitiesOphtho criteria for CZSMacular scarring and focal pigmentary retinal mottlingCongenital contractures (Arthrogryposis)Congenital contractures (Arthrogryposis)Marked early hypertonia and symptoms of extrapyramidal involvementOther Known Abnormalities Associated with Zika Virus SyndromeOther Ocular Findings: StrabismusCutis GyrataHypotoniahyperreflexiaseizuresSummary: Zika- Associated Adverse Pregnancy OutcomesQuestions?